Shigeto Fuse

Pulse wave velocity and ankle brachial index in patients with Kawasaki disease

Abstract Background : Pulse wave velocity (PWV) and ankle brachial index (ABI) are a useful method to estimate atherosclerosis in adults, while a history of Kawasaki disease (KD) may be a risk factor for later atherosclerosis of systemic arteries. PWV and ABI in patients with a history of KD have not been reported.

Hemolysis complicating coil occlusion of patent ductus arteriosus

We report on 5 patients who developed hemolysis (the Hemolysis group) following coil occlusion for PDA, and compare their data to 66 cases which were not complicated by hemolysis despite residual leak (the No Hemolysis group). A significant leak with a heart murmur was more frequent in the Hemolysis group than in the No Hemolysis group. The ratio of the sum of the loop diameter of coils to the minimal diameter of the ductus (C/D) in those who developed persistent hemolysis that needed a second intervention (2.2+/-0.4) was significantly smaller than in the No Hemolysis group (3.1+/-1.1). Persistent hemolysis can occur if a significant residual shunt remains after implantation of coils with small C/D. As this complication may be avoided by complete closure or, if not complete, a minimal leak, we should be careful to make residual leaks as small as possible by the use of multiple coils.

An infant with Costello syndrome complicated with fatal hypertrophic obstructive cardiomyopathy.

We report a 3‐month‐old girl with Costelio syndrome complicating fatal hypertrophic obstructive cardio‐myopathy. She had typical findings of this syndrome, slight dyspnea and persistent wheezing. Doppler echocardiography revealed asymmetric septal hypertrophy and systolic anterior movement of the anterior mitral leaflet. There was grade 1 mitral regurgitation. Although once her heart failure had been controlled medically, she died suddenly following deterioration of her heart condition. Costelio syndrome can complicate fatal hypertrophic obstructive cardiomyopathy.

Standard method for ultrasound imaging of coronary artery in children.

The Child Coronary Arterial Diameter Reference Study Group of the Japan Kawasaki Disease Society recommends ultrasound imaging as the standard method for measuring the diameter of the coronary artery in children. The patient is examined in a supine or right decubitus position by using a sector probe (≥5 MHz). The coronary arterial diameter measured at the minimum gain setting is the distance between the internal echo edge and the internal echo edge. The diameter is measured during the early diastolic phase at the end of the T wave. The left main coronary artery and the proximal right coronary artery are approached from the precordial short axis at the level of the aortic valve. The proximal and mid‐right coronary arteries are observed on the atrioventricular groove, anterior to the tricuspid valve ring. The right coronary artery of the acute margin of the heart runs along the right side of the tricuspid valve ring. The distal right coronary artery is observed on the posterior atrioventricular groove, and the posterior descending branch of the right coronary artery is observed on the posterior interventricular groove. The right coronary artery is also well observed from the right sternal border in the right decubitus position. Proximal and mid‐anterior descending arteries are observed on the anterior interventricular groove. The proximal left circumflex coronary artery is observed in the atrioventricular groove, anterior to the mitral valve ring.

Rotavirus encephalitis and cerebellitis with reversible magnetic resonance signal changes

administration of a liquid valganciclovir formulation. Clin. Pharmacol. Ther. 2007; 81: 867–72. 15 Galli L, Novelli A, Chiappini E et al. Valganciclovir for congenital CMV infection: A pilot study on plasma concentration in newborns and infants. Pediatr. Infect. Dis. J. 2007; 26: 451–3. 16 Lombardi G, Garofoli F, Villani P et al. Oral valganciclovir treatment in newborns with symptomatic congenital cytomegalovirus infection. Eur. J. Clin. Microbiol. Infect. Dis. 2009; 28: 1465–70. 17 Nassetta L, Kimberlin D, Whitley R. Treatment of congenital cytomegalovirus infection: Implications for future therapeutic strategies. J. Antimicrob. Chemother. 2009; 63: 862–7.

High dose of intravenous antithrombin III without heparin in the treatment of disseminated intravascular coagulation and organ failure in four children

In several animal experiments, high doses of antithrombin III concentrates have shown beneficial effects on mortality and reversal of coagulation abnormalities which had resulted from disseminated intravascular coagulation. Other experiments have suggested that antithrombin III infusion without heparin is effective in the treatment of organ failure. We clinically treated children suffering disseminated intravascular coagulation only with antithrombin concentrate. Four patients suffering disseminated intravascular coagulation with organ failure were selected. We started antithrombin III concentrate infusion as soon as the diagnosis was established. The dosage of antithrombin III was 120–250 units/kg/day for 2 or 3 days. Heparin was not used. All 4 patients recovered completely and quickly without any complications within 14 days. We suggest that the high‐dose antithrombin III infusion without heparin is an effective and safe therapy for disseminated intravascular coagulation with organ failure.

Clinical characteristics and computed tomography findings in children with 2009 pandemic influenza A (H1N1) viral pneumonia

Abstract In this article we review the clinical characteristics and computed tomography (CT) findings in children with 2009 pandemic H1N1 influenza viral pneumonia. The medical charts of 88 children with pandemic H1N1 influenza virus infection, admitted to our hospital in Japan from 10 August to 28 December 2009, were reviewed; we compared the clinical features of these children with those of 61 children admitted with seasonal influenza A during the previous 3 seasons. Of 88 patients, 53 (60%) had radiographic findings consistent with pneumonia and 34 patients underwent a chest computed tomography (CT) scan. Pneumonia was a more frequent complication in children with pandemic H1N1 influenza compared with those with seasonal influenza (60% vs 11%; p < 0.001). The predominant CT findings were unilateral or bilateral multifocal consolidation (15/34; 44%) associated with ground-glass opacities in the peribronchovascular region. The second most common CT finding was unilateral diffuse consolidation or atelectasis in 1 or more lung zones (12/34; 35%). The chest CT findings of unilateral or bilateral multifocal consolidation often associated with ground-glass opacities were commonly seen in children with pandemic H1N1 influenza viral pneumonia. Atelectasis was seen in patients who required oxygen administration.

Development of a reexpandable covered stent for children.

The use of covered stents in children is limited by the need for a large long sheath for delivery and the impossibility of redilation once implanted. The authors developed a reexpandable covered stent implantable in children through a small sheath and evaluated its clinical feasibility in mini piglets. An original Palmaz stent was covered with a polyurethane membrane that could be stretched up to 700%. Under general anesthesia, the authors implanted the covered stents in six mini piglets using a long sheath with a diameter approximately 1 French larger than the recommended size required to deliver an uncovered Palmaz stent. The implantation technique was similar to conventional stent implantation. In six piglets, the stent could be redilated from 7.5 to 8.7 mm 28–70 days after implantation. Macroscopic and microscopic examination showed intimal coverage of the coating with minimal inflammatory reaction around the stent. Our newly designed reexpandable stent covered with a polyurethane membrane is promising for use in children.

The Throat Flora and Its Mitogenic Activity in Patients with Kawasaki Disease

The etiology of Kawasaki disease (KD) remains unknown, although some infectious organism has been suggested as the cause. Recent studies suggest that some bacterial toxins with superantigen activity are involved in its pathogenesis, but no specific bacterial toxin has yet been identified. Throat swabs for bacterial culture were obtained from 21 patients with KD and 20 with other febrile illnesses as controls. Mitogenic activity in culture supernatants obtained from individual bacterial strains was measured by lymphocyte proliferation assay. Sixty‐one bacterial strains were isolated from KD patients, and 62 strains from control patients. There was no apparent difference in bacterial species in the throat flora between KD patients and febrile controls. Moreover, total and individual mitogenic activity of strains from KD patients was no greater than that of strains from febrile controls. The bacterial superantigen activity of throat flora may not play a major role in the pathogenesis of KD.

Double outlet right ventricle with intact ventricular septum

We report a rare case of DORV with an intact ventricular septum (intact VS). The patient was a 2-day-old boy, born at 39 weeks and weighing 3090 g, who was referred to the Department of Pediatrics, Sapporo Medical University Hospital, Sapporo, Japan, because of a heart murmur. He had cyanosis and a systolic murmur at the lower intercostal spaces. Chest X-ray showed cardiomegaly (cardiothoracic ratio, 67%) and the electrocardiogram revealed normal sinus rhythm, right axis deviation and right ventricular hyper trophy. Echo cardiography demonstrated situs solitus, normal relationship of great arteries, side by side, bilateral conus, intact VS, persistent foramen ovale, pulmonary stenosis and severe mitral valve insuffi ciency ( Fig. 1a,b). We performed balloon atrial septostomy when he was 1 month old, and he underwent a left modifi ed Blalock – Taussig shunt at 5 months. Cardiac catheterization was performed when he was 18 months old. His left ventricular pressure was extremely low, 10/4 mmHg, the diastolic volume was 11 mL, 48% of normal, and the ejection fraction was 76%. Left ventricular angiography showed a smooth and thin wall of the left ventricle, resembling Uhl’s disease. Both right and left coronary artery were normally demonstrated ( Fig. 2). Right ventricular pressure was 92/11 mmHg with a pulmonary artery Patient Report